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Idiopathic cerebellar ataxia associated with celiac disease: lack of distinctive neurological features
  1. Department of Neurology, Royal Hallamshire Hospital, Glossop Road, Sheffield S10 2JF, UK
  1. Dr G A B Davies-Jones, Department of Neurology, Royal Hallamshire Hospital, Glossop Road, Sheffield S10 2JF, UK.
  2. R SCALA,
  3. A FILLA,
  1. Department of Neurological Sciences, Via S Pansini 5, 80131 Naples, Italy

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    Although applauding the contribution of Pellecchiaet al 1 to the more widespread recognition of the association between gluten sensitivity and ataxia we disagree that ataxia associated with gluten sensitivity lacks “distinctive neurological features”. Both their data and our own2 indicate that this group of patients can be distinguished by the late (non-childhood) onset of gait ataxia with relatively mild upper limb signs, analogous to Harding’s group 1.3 Again, coexistent neuropathy is common in these patients, found in two out of three of the patients of Pellecchiaet al and 21 of our 28.2 We agree that gastrointestinal symptoms are rare: rather than entitling their paper “lack of distinctive neurological features”, perhaps “lack of distinctive gastroenterological features” might have been more appropriate!

    We were surprised at the high specificity and sensitivity of increased antigliadin antibody titres in their hands. Although we found both IgA and IgG antigliadin antibodies to be invaluable screening tools in patients with ataxia, only 11 of our 28 patients with increased antigliadin antibodies had histology of overt coeliac disease on duodenal biopsy, the remainder having normal or non-specific inflammatory changes but with an HLA genotype in keeping with gluten sensitivity. It is interesting to note that despite the often quoted high sensitivity for coeliac disease of increased antiendomysium antibody titres, such was found in only one of three patients of Pellecchia et al with coeliac disease. This concurs with our impression of very modest sensitivity of antiendomysium antibodies in gluten ataxia.

    Gluten sensitivity is common in patients with ataxia, and can be identified by increased antigliadin antibody titres in the presence of appropriate histocompatibility antigens2. Although the clinical features of gluten ataxia are not entirely specific, they are distinctive.


    Pellecchia et al reply:

    We thank Hadjivassiliou et al for their interesting comments on our paper. They suggest that patients with gluten ataxia can be distinguished by the late onset of gait ataxia and the relatively mild upper limb signs. Our results support the finding of a late onset in these patients, but this feature cannot be considered a distinctive one. In fact, in our population 11 out of 24 patients with idiopathic cerebellar ataxia had a late onset, but only three of them were affected by celiac disease.1-1Furthermore, we do not think that celiac patients may be distinguished by mild upper limb signs and coexistent neuropathy; in our study 20 out of 24 patients with idiopathic cerebellar ataxia, including the three patients with celiac disease, had ataxic gait as the presenting and prominent clinical feature. Similarly, nerve conduction studies, performed in 17 out of 24 patients, showed a peripheral neuropathy in nine, including two out of the three patients with celiac disease.1-1

    We understand that some discrepancies arise comparing our study with that of Hadjivassiliou et al. Firstly, only six out of their 28 patients had evidence of cerebellar atrophy on MRI,1-2 whereas all of our patients had cerebellar atrophy. Secondly, many of their patients had a peripheral neuropathy in the absence of cerebellar atrophy.1-2 This finding could explain the relatively mild upper limb signs. Although two of our three celiac patients had a clinically silent peripheral neuropathy, we think that their ataxia was explained by cerebellar atrophy. Thirdly, we found a high prevalence (12.5%) of celiac disease on duodenal biopsy among patients with idiopathic cerebellar ataxia,1-1 whereas none of the six patients with cerebellar atrophy described by Hadjivassiliouet al showed histological features of celiac disease.1-2 It would be interesting to know the prevalence of gluten ataxia among all ataxic patients screened for antigliadin by Hadjivassiliou et al.

    Our series is too small to estimate the sensitivity of both antigliadin and antiendomysium antibodies in gluten ataxia; unfortunately Hadjivassiliou et al did not report any data on antiendomysium antibody screening in their patients. On the other hand, we were surprised at the high prevalence of antigliadin antibody positivity (12%) in the normal population studied by Hadjivassiliouet al in a previous report.1-3This is by contrast with the 2% of antigliadin antibody positivity found in a large population by Catassi et al.1-4 Further studies are required to better characterise the syndrome of cerebellar ataxia associated with celiac disease or gluten sensitivity.


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